Medulloblastoma is also known as or subsumes Primitive neuroectodermal tumor of the posterior fossa. -ed.
In this clinical article, Dr. Roger Packer of Children's National Medical Center reviews medulloblastoma, the most common subtype of childhood malignant brain tumor. Survival has risen over the past quarter century for patients with medulloblastoma, and despite the malignant nature of the tumor, up to 90% of older children with nondisseminated disease at the time of diagnosis can be effectively treated, many of whom will be cured. Treatment is not as effective for children with disseminated disease or those who are young and are likely to develop severe sequelae from the craniospinal radiation required to cure disease. Chemotherapy has a definite role in the management of medulloblastoma. Total or near-total surgical resections have been associated with the best survival rates but may result in significant permanent neurologic side effects in up to one quarter of all patients, primarily in the posterior fossa mutism syndrome. The recent biological understandings of medulloblastoma are being incorporated into patient stratification scheme and will soon be a part of treatment regimens for patients with both newly diagnosed and recurrent disease.